NURSING CARE OF THE CHILD BORN WITH A

PHYSICAL OR DEVELOPMENTAL CHALLENGE

CASE:
Mia Sparrow, 22 years old, is a new mother whose newborn has been admitted to the
neonatal intensive care unit for care of a neural tube disorder and developmental dysplasia of the
hip. Ms. Sparrow is obviously upset over the diagnosis. She has not named the baby, and her
parents have not visited.
She says to you, I’m a good person. The only thing I did wrong during pregnancy was to
take some cough medicine. How could this have happened to me?”
How would you answer this mother? What type of advice and support does she need?

KEY TERMS
Achondroplasia Fistula Polydactyly
Ankyloglossia Frenulum Spina Bifida
Atresia Gastroschisis Stenosis
Cleft Lip Glossoptosis Syndactyly
Cleft Palate Hydrocephalus Talipes
Craniosynostosis Meconium Plug Torticollis
Developmental Dysplasia Of The Omphalocele Transillumination
Hip (Ddh) Pectus Excavatum Volvulus

OBJECTIVES

After mastering the contents of this chapter, you should be able to:
1. Describe common physical and developmental disorders that occur in newborns, infants, and children.
2. Assess newborns who are born physically or developmentally challenged.
3. Formulate nursing diagnoses for newborns born with a physical or developmental challenge.
4. Establish expected outcomes to meet the needs of the child with a physical or developmental challenge and
assist parents to manage transitions across differing healthcare settings.
5. Implement nursing interventions for care of a newborn born with a physical or developmental challenge,
such as preventing infection in a child with a neural tube disorder.
6. Evaluate expected outcomes to determine achievement and effectiveness of care.
7. Integrate knowledge of congenital physical or developmental challenges with the interplay of nursing
process; the six competencies of Quality & Safety Education for Nurses (QSEN), including Patient-
Centered Care, Teamwork & Collaboration, Evidence-Based Practice (EBP), Quality Improvement (QI),
Safety, and Informatics; and Family Nursing to promote quality maternal and child health nursing care.
8. Assist with achieving 2020 National Health Goals related to newborns born physically or developmentally
challenged.

When a newborn is born with an apparent physical or developmental challenge, nurses play a major role in
supporting and educating the parents to promote bonding.

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 With some newborns, their congenital disorder may be corrected with surgery, and they will have no long-
term sequela.

With other newborns, the congenital disorders may require long-term care even with surgical correction.
This chapter covers physical disorders of the skeletal, gastrointestinal, and neurologic systems that are apparent at
birth or recognized soon after.

FOR CARE OF A NEWBORN WHO IS PHYSICALLY OR DEVELOPMENTALLY CHALLENGED

ASSESSMENT

The following eight primary needs of newborns should be assessed for signs that they are being established
considering the newborn’s physical and developmental challenges:
1) Adequate respiration
2) Extrauterine circulation
3) Body temperature stabilization
4) Blood sugar stabilization
5) Prevention of infection
6) An infant-parent bond. Adequate stimulation
7) Ability to take in adequate nutrients
8) Ability to achieve waste elimination

NURSING DIAGNOSIS

1. Imbalanced nutrition, less than body requirements, related to inability to take in adequate nutrition
secondary to a physical challenge
2. Impaired physical mobility related to congenital anomaly
3. Risk for impaired parenting related to the birth of child with a congenital anomaly
4. Anticipatory grieving (parental) related to loss of the idea of the perfect child

OUTCOME IDENTIFICATION AND PLANNING

1) Nurses play an important role in providing immediate care to high-risk newborns at birth as well as
stabilizing them until the pediatric team arrives to assume care or transport the newborn to a high-risk
nursery. Consideration of the family’s resources, both emotional and financial, is an important aspect of
planning care and establishing expected outcomes (Nes, Rysamb, Hauge, et al., 201 4).

2) It is important to consider both the short- and long-term needs of the newborn and how these needs may
affect the family. Supportive family members can be a critical asset to parental adjustment (Carmichael,
Ma, Tinker, et al., 2014).

3) Providing nursing support as part of an interdisciplinary team, including social workers, therapists,
nutritionists, medical specialists, and other community resources can also help with parental adjustment.

4) Refer parents to helpful websites and other resources when appropriate

IMPLEMENTATION
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 1. Nursing interventions for a newborn who is physically challenged include immediate life-sustaining
measures such as providing oxygen or adequate intake of nutrients when a disorder prevents the infant from
establishing respirations or sucking.

2. Encouraging skin-to-skin contact and interacting with the newborn promotes infant- parent bonding.

3. Educating the parents about procedures the infant may undergo lessens the parental anxiety and enhances
self-esteem.

4. Parents experience the same stages of grief: denial, anger, bargaining, depression, and adjustment as those
whose child has died at birth .
5. It is important for the nurse to provide positive role modeling when caring for the emotional and physical
needs of the newborn; it helps the parents to adjust to parenting a child born with a physical or
developmental challenge.

OUTCOME EVALUATION

Outcome evaluation should focus on establishing expected outcomes for the child’s physical and
developmental health needs as well as the family’s coping ability for current and future health of the child. This
includes addressing the family concerns and providing resources to support the family during and after discharge.

Examples of expected outcomes may include:

✔ Parent describes positive features of child by 2 weeks.

✔ Parents state they are comfortable with enteral feeding by 1 month.

✔ Child is ambulatory with walker or wheelchair by 2 years of age.

CARE AT BIRTH OF THE NEWBORN WHO IS PHYSICALLY OR DEVELOPMENTALLY

CHALLENGED

The pediatric provider or neonatologist typically provides the parents with medical information regarding the
health status of their newborn.

This information should be provided to the parents as quickly and accurately as possible

The nurse can provide support to the family by keeping them informed of their newborn’s health status and
facilitating their interaction with the healthcare team.

It is distressing for parents, who expect to hold their newborn immediately after birth, to watch their newborn
undergo a medical evaluation and medical intervention while physically separated from their newborn.

NURSING CARE PLANNING TO RESPECT CULTURAL DIVERSITY

When the parents are with their newborn, the nurse can begin by describing the newborn’s physical condition,
related to the diagnosis, to the parents.

Medical equipment and its purpose should also be explained.

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 Parents should be given the opportunity to ask questions and interact with their newborn.
Comments by the nurse related to normal newborn observations can assist the parents in relating their newborn.

A typical explanation for Mia, for example, might be, “When your doctor placed your baby on your
abdomen, you might have noticed that your baby’s spinal cord isn’t completely formed, something called a
meningocele.

“Although that could be more extensive, at first inspection, it seems to be a problem that can be repaired.
Her hips may also need some treatment.”

“ I’ll bring the baby’s Isolette over so you can see her. Notice how bright-eyed and alert she is for just
being born.” (Referring to the newborn by their name is helpful to personalize the interaction.

PHYSICAL AND DEVELOPMENTAL DISORDERS OF THE SKELETAL SYSTEM

Either genetic or environmental factors can compromise fetal physical growth to such an extent that they result in
skeletal disorders in the newborn.

1. ABSENT OR MALFORMED EXTREMITIES

Congenital skeletal disorders can result from reasons/factors such as:
⮚ maternal drug ingestion

⮚ virus invasion

⮚ amniotic band formation in utero.

Cause of the anomaly is unknown.

Children born without an extremity or with a malformed extremity can be fitted with a prosthesis as
early in life as about 6 months so the infant can learn to stand at the normal time or handle and explore objects
readily.

However, it may be adventitious to allow the child to grow and learn to use their altered body or limb
without a prosthesis.

Introducing a prosthesis early in life may prevent a child from adjusting to a missing extremity, for
example, such as learning to write with their feet or sliding across the floor rather than walking.

Often, parents and therapists will teach children in therapy to function both with and without a prosthesis.

Children are resilient and can become so proficient at these adjustments in their born deformity that later
in life they may not see any advantage to using a prosthesis. Those affected by a skeletal anomaly can choose to
use a prosthesis as an adult or not.

Depending on the condition, in many children, there is a potential for better function if the malformed
portion of an extremity is amputated before a prosthesis is fitted. This creates a difficult decision for parents
because it is one they cannot undo later. They need assurance that hands with malformed fingers, for example,
will not later grow to become normal and that a well-fitted prosthesis will allow their child a more usual
childhood and adult life than if the original disorder was left unchanged.

Learning to use a hand prosthesis takes weeks to months, and it also involves therapy.

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 It helps if parents can think of interesting activities when introducing the prosthesis so the child can
immediately see how useful it will be to use.

Gait training for the use of lower extremity prostheses begins with the use of parallel bars and proceeds to
independent walking and mastery of steps. Again, suggesting activities the child needs to walk to do offers
motivation for trying to use the prosthesis.

Children who are born with an absent extremity may need help not only in mastering the use of a
prosthesis but also in forming a positive body image of themselves as whole. If possible, in the newborn period,
introduce parents to the rehabilitation team who will be following their child. Further steps will then be outlined
to help them move past the helplessness they may be feeling to more positive actions. Visiting with a child who
uses a prosthesis well can be a great help in convincing parents that their child can lead a normal life. Young
children with a congenital extremity may not grieve over the lost extremity as do adults or older children.

2. FINGER AND TOE CONDITIONS

Finger or hand deformities occur in about 3% of all births.

POLYDACTYLY is the presence of one or more additional fingers or toes.

When an entire extra finger or toe forms, the supernumerary digit is usually amputated in infancy or early
childhood.

These extra fingers are often just cartilage or skin tags, and removal is simple and cosmetically sound.

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SYNDACTYLY (two fingers or toes are fused), the fusion is usually caused by a simple webbing
Separation of the digits into two sound and cosmetically appealing ones is usually successful.

In other instances, the bones of the fingers or toes are also fused, and cosmetic appearance and function cannot be
fully reconstructed (Sullivan & Adkinson, 2016).

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 These digit anomalies are always upsetting to parents (one of the first things new parents do is count the
fingers and toes of newborns) and may cause them to view their infant as defective rather than being an infant
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with a simple anomaly. Encourage them to air their feelings and concerns as they deliver the news to family and
friends. Because hands are so important for writing, self-care, or computing, they may need reassurance at
health maintenance visits for the first few years of their child’s life that the child is perfect in other ways so they
can accept and help the child develop self-esteem.

Children may need this same type of assurance as they grow older so they can think of themselves as
well people. Often, identifying a skill or talent that the child excels in can provide another avenue for
developing self-esteem. There are many examples of children with one limb or a deformity playing sports, such
as swimming, excelling in the arts, or playing musical instruments. If a child expresses a desire to learn these
activities, allowing the child to participate (if medically safe and sound) typically leads to profound beneficial
outcomes for all.
CHEST DEVIATIONS
Pectus excavatum, or Funnel chest, it is an indentation of the lower portion of the sternum. It is the most
common congenital deformity of the anterior chest, occurs in about 1 out of 500 live births, and affects boys 4
times more often than girls. The concern may not be present at birth but becomes more obvious as the child
grows to school age or adolescence. As a result of the deformity, lung volume is apt to be decreased and the
heart is displaced to the left. The condition can be repaired, for either cosmetic reasons or physiologic reasons,
such as to expand lung volume (Abdullah &Harris, 2016). \

With pectus carinatum, the sternum is displaced anteriorly, increasing the anteroposterior diameter of the
chest. This anomaly can be surgically corrected (Tikka,Kalkat, Bishay, et al., 2016).
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TORTICOLLIS (WRY NECK)

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Torticollis is a term derived from the terms tortus (twisted) and collum (neck). Torticollis (wry neck) occurs as
a congenital anomaly when the sternocleidomastoid muscle is injured and bleeds during birth (Ryu, Kim, Kim,
et al., 2016). This tends to occur in newborns with wide shoulders when pressure is exerted on the head to
deliver the shoulder either with a vaginal or cesarean birth. The infant holds the head tilted to the same side as
the muscle that is involved; the chin rotates to the opposite side. The injury may not be noticeable in the
newborn and may become evident only as the original hemorrhage recedes and fibrous contraction occurs at 1
to 2 months of age. A thick mass over the muscle can usually be palpated at that time.

To relieve torticollis, parents need to begin a program of passive stretching exercises and therapy, laying the
infant on a flat surface and rotating the head through a full range of motion. Often, pediatric physical therapists
are involved in the home therapy treatment for infants with torticollis. In addition, parents should always
encourage the infant to look in the direction of the affected muscle. They can encourage this by holding the
child to feed in such a position that the child must look in the desired direction. Placing a mobile on the child¡¯s
crib can encourage the child to look toward the affected side. Speaking to and handing the child objects from
the affected side is another helpful exercise.

If manual stretching is begun early and performed consistently by parents, further treatment usually is not
necessary. If extreme injury to the muscle occurred, torticollis can lead to the continued elevation of one
shoulder. Although a rare complication, this has the potential to lead to scoliosis later in life. Therefore, help
parents to understand that their actions are important therapy, not just games. Otherwise, the exercises seem so
simple parents may not take them seriously. In the few instances in which simple exercises are not effective and
the condition still exists at 1 year of age, surgical correction followed by a neck immobilizer may be necessary.

CRANIOSYNOSTOSIS

Craniosynostosis is the premature closure of the sutures of the skull. This may occur in utero or early in infancy
because of rickets or irregularities of calcium or phosphate metabolism; it also occurs as a dominantly inherited
trait and occurs more often in boys than in girls (Children’s Craniofacial Association, n.d.). Measuring the
infant’s head circumference during the first 18 months of life is advocated by the American Academy of
Pediatrics (AAP; Mulpuri, Song, Gross, et al., 2015).

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This condition needs to be detected early because premature closure of the suture line will close the fontanelles,
seal the skull closed, and compromise brain growth. If the sagittal suture line is the one that closes prematurely,
the child’s head tends to grow anteriorly and posteriorly. If the coronal suture line fuses early, the orbits of the
eyes become misshapen and the increased intracranial pressure may lead to eye disorders such as
exophthalmos, nystagmus, papilledema, strabismus, and atrophy of the optic nerve with consequent loss of
vision. Premature closure of the coronal suture line is associated with syndactyly. Therefore, make a point at
well-child assessments to observe the head circumference for all infants, especially those with syndactyly.
Cardiac anomalies, choanal atresias, or disorders of elbows and knee joints can also be associated.

Craniosynostosis is diagnosed by X-ray or ultrasound, which reveals the fused suture line. If the suture line
involved is the sagittal, treatment may involve only careful observation; if the coronal suture line is involved, it
will need to be surgically opened to prevent brain compression and an abnormally shaped head by 9 to 12
months (Jubbal, Agrawal, & Hollier, 2017).

ACHONDROPLASIA

Achondroplasia (chondrodystrophia) is a failure of bone growth inherited as a dominant trait, which causes a
disorder in cartilage production in utero. The epiphyseal plate of long bones cannot produce adequate cartilage
for longitudinal bone growth; this results in both arms and legs becoming stunted (Ornitz & Legeai-Mallet,
2017).

Because the bones of the cranium are of membranous origin, the head continues to grow normally, causing
children’s heads to appear unusually large in contrast to their extremities. The forehead is particularly
prominent and the bridge of the nose becomes flattened. Children’s trunks are of near-normal size, but a
thoracic kyphosis (outward curve) and lumbar lordosis (inward curve) of the spine may develop. Because this is
a cartilage, not a brain growth concern, gross motor development may be slowed, but intelligence is not
affected.

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 Achondroplasia can be diagnosed in utero by ultrasound or at birth by X-ray by comparing the length of
extremities to the usual length (in the average child, the arms can be extended to the distance of the midthigh).
An X-ray will also reveal characteristic abnormally flaring epiphyseal lines. Children with achondroplasia
rarely reach a height of more than 4 ft 6 in. (140 cm). Women with this condition may have difficulty with
childbearing because of a small pelvis, generally necessitating a cesarean birth.

Children with achondroplasia become aware of their appearance as early as the preschool years. They are apt to
become acutely aware of their appearance during school age, when they realize they look so different from
other children. In order to help them grow, they may be prescribed growth hormone, or although controversial,
leg lengthening may be possible (Ornitz & Legeai-Mallet, 2017). Ideally, such children have parents who have
helped them adjust well to their short stature as well as help them develop good self-esteem so they can be
happy in their body, no matter what is their final height.

Children need to be informed as they reach adolescence that, as with all dominantly inherited disorders, there is
a high probability their children will inherit the disorder. This can make adolescence a particularly difficult
time for these children as they realize both some occupational and reproductive options may be limited for
them. Continued guidance or counseling can help them to emerge from this period with feelings of high self-
esteem as adults.
TALIPES DISORDERS

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The word talipes is formed from the Latin talus (ankle) and pes (foot). The talipes deformities, therefore, are
ankle and foot disorders, popularly called clubfoot. The term clubfoot implies permanent crippling to many
people, and because this is no longer true with effective surgery, avoid using the term when discussing talipes
disorders with parents. Concerns that may remain after surgery include that the child’s right and left shoe size
may vary and the child may have asymmetry of leg length.

Approximately 1 child in every 1,000 is born with a talipes disorder, and it occurs more often in boys than in
girls. It probably is inherited as a polygenic pattern, and it usually occurs as a unilateral problem (Sanzarello,
Nanni, & Faldini, 2017).

Some newborns who appear to have a talipes disorder actually have only an unusual foot position (a
pseudotalipes) that developed because of their cramped intrauterine position. In these infants, the foot can be
brought into a straight position by manual manipulation. In a true disorder, the foot cannot be properly aligned
without further intervention. Be certain to demonstrate to parents that, if a pseudodisorder is present, the foot
can easily be brought into line or is not deformed. Otherwise, the first time parents fit booties or shoes on the
infant, they may notice this odd position and worry the foot is misshapen when it is not. Stretching the foot into
line every day will solve the problem in a short time.

A true talipes disorder can be one of four separate types: plantar flexion (an equinus or horse foot position, with
the forefoot lower than the heel); dorsiflexion (the heel is held lower than the forefoot or the anterior foot is
flexed toward the anterior leg); varus deviation (the foot turns in); or valgus deviation (the foot turns out). Most
children with talipes deformities have a combination of these conditions or have an equinovarus (Fig. 27.3A) or
a calcaneovalgus disorder (a child walks on the heel with the foot everted).

Casts for bilateral equinovarus.

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 Assessment

The earlier a true disorder is recognized, the better will be the correction. Make a habit, therefore, of
straightening all newborn feet to the midline as part of the initial assessment to detect this disorder. If there is a
possible questionable deformity, refer to the pediatric physician and orthopedist specialist to begin the process
of evaluating the infant properly.

Therapeutic Management

Correction is achieved best if it is begun in the newborn period. For correction, a series of casts or braces are
applied to gradually mold the foot into good alignment (a Ponseti method) (Sanzarello et al., 2017). Although
the disorder involves the ankle, the cast or brace extends above the knee to ensure a firm correction. Because
talipes casts are high on the leg, change diapers frequently to prevent a wet diaper from touching the cast and
causing it to become soaked with urine or meconium. Review with parents how to check the Infant’s toes for
coldness or blueness and how to blanch a toenail bed and watch it turn pink to assess for good circulation.
Because a newborn cannot report pain except by generalized crying, they must evaluate crying episodes in the
infant carefully. Such crying may be because of colic, hunger, or wet diapers, or it might also be because of the
tingling feeling of circulatory compression (as when a foot is at sleep) from too tight a cast.

Infants grow so rapidly in the neonatal period that casts or braces for talipes deformities must be changed or
adjusted almost every 1 or 2 weeks. If a mother has a complication or is exhausted from childbirth, be certain
she will be able to make arrangements for another family member or friend to bring the infant to the hospital
for frequent cast changes or brace adjustments.

After approximately 6 weeks (the time varies depending on the extent of the problem), the final cast will be
removed. Following this, the infant may have to sleep in Denis Browne splints (shoes attached to a metal bar to
maintain position) or high-top shoes at night for a few more months to ensure an effective correction. Parents
may need to perform passive foot exercises such as putting the infant’s foot and ankle through a full range of
motion several times a day for several months. These seem to be simple maneuvers, so be certain to stress their
importance to the parents; otherwise, they are easy exercises to omit when people¡¯s lives are busy. Pediatric
physical therapists are often part of the interprofessional therapy team to assist in rehabilitation at the hospital
and at home.

Although a successful correction cannot be guaranteed, the prognosis for a full correction is good. For children
who do not achieve correction by casting, additional surgery is yet another option to achieve a final correction.

DEVELOPMENTAL DYSPLASIA OF THE HIP

Developmental dysplasia of the hip (DDH) (often referred to as congenital hip dysplasia) is improper formation
and function of the hip socket and is considered a spectrum of abnormalities affecting the hip joint. DDH is a
fairly common musculoskeletal condition found in newborns, with the prevalence estimated to range from 1.6
to 28.5 per 1,000 infants, averaging about 5% of newborns who have some radiographic abnormality of the hip
(Shaw & Segal, 2016).

DDH is a leading cause of orthopedic disability in childhood and adult life because it can lead to premature
arthritis requiring hip replacement (the disorder is responsible for up to 28% of hip replacements in people under
60 years of age) (Shaw & Segal, 2016). The disorder may be evident as either subluxation or dislocation of the

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head of the femur

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With the disorder, the acetabulum of the pelvis is unusually flattened or shallow. This prevents the head of the
femur from remaining in the acetabulum and rotating adequately. In a subluxated hip, the femur ¡°rides up¡±
because of the flat acetabulum; in a dislocated hip, the femur rides so far up it actually leaves the acetabulum.
Why the disorder occurs is unknown, but it may be from a polygenic inheritance pattern. It may also occur from
a uterine position that causes less-than-usual pressure of the femur head on the acetabulum.

DDH is more likely to occur with breech birth, a female infant, and a mother’s first pregnancy (Jackson, Runge,
& Nye, 2014). It is usually unilateral and found 6 times more frequently in girls than in boys, possibly because
the hips are normally more flaring in females and possibly because the maternal hormone relaxin causes the
pelvic ligaments to be more relaxed during pregnancy, which causes the femur to not press as effectively into
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the acetabulum during intrauterine life, thus deepening the space. Additional risk factors include family history
of DDH, oligohydramnios, large birth weight for gestational age, metatarsus adductus, and torticollis (Shaw &
Segal, 2016). Current literature suggests that the natural history to delineate the reason for the DDH appears to
be dependent both on the type and severity of the hip abnormality and may be the combination of risk factors
presented (Mulpuri et al., 2015).

Assessment

All infants should be screened for DDH from birth and up until 3 months of age by performing the Ortolani &
Barlow maneuver (Shaw & Segal, 2016). Detecting developmental dysplasia of the hip in the newborn is
important because the longer the condition goes undetected, the more difficult it is to correct. On inspection, the
affected leg may appear slightly shorter than the other because the femur head rides so high in the socket. This
is most noticeable when the child is lying supine and the thighs are flexed to a 90-degree angle toward the
abdomen, causing one knee to be lower than the other (a Galeazzi sign; ). An unequal number of skin folds
may also be present on the posterior thighs.

This finding is unreliable, however, because some infants with normal hips have an uneven number of posterior
thigh skin folds. Subluxated or dislocated hips are best assessed by noting whether the hips abduct

Signs of developmental dysplasia of the hip (A) With the child in a supine position, the right knee on the side of
the subluxation appears lower than the left because of malposition of the femur head. (B) Asymmetry of skin
folds and prominence of the trochanter on the right side.

ASSESSING ORTOLANI AND BARLOW SIGNS

Purpose: To assist in detecting developmental dysplasia of the hip

Procedure Principle

1. Lay the infant supine and flex the knees 1. Proper positioning helps ensure
to 90 degrees at the hips. accurate results.

2. Place your middle fingers over the 2. Placing your fingers in this way
greater trochanter of the femur and your allows for abduction of the hips.
thumb on the internal side of the thigh

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over the lesser trochanter

3. Abduct the hips while applying upward 3. Normally, no sound is heard. A clicking or
pressure over the greater trochanter, and clunking sound is a positive Ortolani sign
listen for a clicking sound. as it occurs when a displaced femoral head
reenters the acetabulum.

4. Next, with your fingers in the same 4. Normally, the hip joint is stable. A feeling of the
position and holding the hips and knees femur head slipping out of the socket
at 90-degree flexion, apply a backward posterolaterally is a positive Barlow sign, which is
pressure (down and laterally) and adduct indicative of hip instability associated with the
the hips. Note any feeling of the femoral developmental dysplasia of the hip.
head slipping

In some infants, the hip abducts properly at a newborn assessment, but at the time of a health maintenance visit
at 4 to 6 weeks, a secondary shortening of the adductor muscles will have occurred, and the disorder will be
first evident. Hip dysplasia may also be difficult to detect at birth in an infant who was born from a breech
presentation because the knees tend to be stiff and not flex readily. Because tight adductor muscles occur in
children with cerebral palsy, these children also need careful assessment. Current guidelines recommend
physical exam screening of all infants up to 6 to 12 months of age at all primary care visits via hip assessment
and continual screening of those with risk factors until walking age (Mulpuri et al., 2015). In regard to
radiologic screening, current guidelines recommend performing an imaging study, such as a hip ultrasound,
before 6 months of age only in infants with one or more of these risk factors: such as breech presentation,
family history, or history of clinical hip instability (Mulpuri et al., 2015). Hip ultrasonography has a good
negative predictive value (as high as 90%) for ruling out DDH (Shaw & Segal, 2016). Generally plain
radiography (X-ray) is not as effective in children less than 6 months of age due to musculoskeletal immaturity
and unreliability to accurately screen for DDH; in children older than 6 months, radiology may be a useful
assessment evaluation tool of treatment progress.

Therapeutic Management

Current recommendations from American Academy of Orthopaedic Surgeons and the AAP (2017) suggest
close monitoring in mild DDH cases because 60% to 80% of clinically identified abnormalities and 90% of
ultrasonographic abnormalities spontaneously resolve without treatment in early infancy (Mulpuri et al., 2015).
In contrast, severe DDH may adversely affect normal hip growth and development and may cause issues into
adulthood, such as osteoarthritis of the hip (Shaw & Segal, 2016). Infants less than 6 months of age are usually
treated by using flexion-abduction splinting devices. Correction of subluxated and dislocated hips involves
positioning the hip into a flexed, abducted (externally rotated) position to press the femur head against the
acetabulum and cause the acetabulum to deepen its contour from the pressure. Brace splints, such as the von
Rosen, Pavlik, Craig, or Frejka, may be utilized for treatment of an unstable hip, and patient and family
preferences should have a substantial influence on which type is chosen (Mulpuri et al., 2015). The Pavlik

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harness is shown to have a high success rate in treating subluxation and reducible DDH. There will be a small
number of children who do not achieve correction by noninvasive methods will have corrective and therapeutic
hip surgery, which may involve having a pin inserted to stabilize the hip.

Nursing Diagnoses and Related Interventions

Nursing Diagnosis: Deficient parental knowledge related to splint, halter, or cast correction for hip dysplasia
Outcome Evaluation: Parents verbalize correct technique for and correctly demonstrate application and
removal of splint or halter device and care of device or cast. A Pavlik Harness. A Pavlik harness is an
adjustable chest halter that abducts the legs. It is the method of choice for therapy because it reduces the time
interval for therapy to 3 to 4 weeks and simplifies care (Fig. 27.6A). Soft plastic stirrups (booties) with quick-
fastening closures attach to leg extension straps and hold the hips flexed, abducted, and externally rotated.
Instruct parents how to lay the infant supine, grasp the infant¡¯s thighs, and abduct them to place the femoral
head into the acetabulum and then apply the harness. The harness is then worn under clothing continually
except for bathing. Advise parents to assess the skin under the straps daily for irritation or redness or potential
skin breakdown from the harness rubbing. Caution them also that the harness achieves its effect by gentle
continual pressure; it will be ineffective if parents remove it frequently or forget to put it in place.

(A) A Pavlik harness (shown over clothing for illustrative

purposes). (B) A hip abduction cast for correction of subluxation of
the hip.

A Spica Cast. If a hip is fully dislocated or the subluxation is severe, an infant may be placed immediately in a
frog-leg, A-line cast, or a spica cast to maintain an externally rotated hip position . These casts are heavy and
are so wide that dressing infants or sitting them in an infant car seat or using a bassinet can be difficult. Be
certain parents have a car seat that can be modified to accommodate a large cast. Like babies with a talipes,
these infants are unable to report a cast is causing circulatory constriction, so they need to be assessed hourly for
circulation to the extremities for the first 24 hours the cast is in place and daily thereafter. Teach parents how to
do this type of neurovascular assessment (check temperature and circulation in toes) before they take an infant
home from the hospital so they can prevent circulatory compression from a rapidly growing leg outgrowing the
cast. Casts will be changed as growth and casting therapy require but maintained for 6 to 9 months. Casting is a
second line of correction because the child is much harder to care for because of the weight of the cast. In some
cases, the joint may become compressed due to the reduction maneuver or tension in the soft tissues around the
hip, blocking the blood supply to the femoral head (avascular necrosis). In its severest form, this can lead to
femoral head death and loss of future growth at the proximal growth plate, causing unequal leg lengths.

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General Care Guidelines. Rarely, but sometimes surgery may still be necessary for a final correction. With
severe hip involvement, some children will be 2 years old before the final cast is removed.

The child and parents will be visiting their orthopedist frequently during these early years. Assess that
they also schedule general health maintenance visits for routine immunizations and overall growth and
development assessment. Spend time during health maintenance visits talking with them about infant
stimulation. Teach parents to hold their child for feeding and to rock and cuddle the infant, even though a large
cast or a brace may be bulky and awkward. Discuss how to bring experiences to the infant because the child
cannot crawl and walk toward interesting objects in the environment. A child¡¯s wagon can supply convenient
and fun transportation. The child may also be able to lie prone and move about on a large skateboard. Many
parents worry the child who is still in a large cast at the normal age for walking (12 months) will never learn to
walk. They can be assured that this is not a problem; when the cast is removed, the child will quickly catch up
with this developmental step. Children who are diagnosed with severe DDH will often have an adjunct therapy
team, such as pediatric physical therapists who are assisting with gross-motor milestone development while the
child is in a brace or recovering from surgery. Nurses must assess that the family is receiving any needed
adjunct therapies, such as physical therapy, and assist with any referrals that are necessary.

Physical and Developmental Disorders of the

Gastrointestinal System
Many of the most common congenital anomalies of the gastrointestinal system, such as cleft lip and cleft palate,
occur because of midline closure failure extremely early in intrauterine life. Others occur because the tract first
forms as a solid tube and then undergoes canalization (hollowing out). At any site where this hollowing out
does not occur, a partial or complete blockage or obstruction will be present. All gastrointestinal disorders can
interfere with an infant¡¯s ability to take in nourishment to some degree at birth.
ANKYLOGLOSSIA (TONGUE-TIE)
Ankyloglossia is an abnormal restriction of the tongue occurring in a small number of newborns caused by an
abnormally tight frenulum, the membrane attached to the lower anterior tip of the tongue (Yoon, Zaghi,
Weitzman, et al., 2017). Tongue-tie (ankyloglossia) is a congenital anomaly in which a tight or shortened
lingual frenulum causes restricted tongue mobility and impaired tongue function (Hazelbaker, 2010). Incidence
rate is estimated to range from 4% to 10% within the newborn population (Yoon et al., 2017). Normally, the
frenulum appears short and is positioned near the tip of the tongue. As the anterior portion of the infant¡¯s
tongue grows, the frenulum becomes located farther back. In most instances, therefore, an infant suspected of
being tongue-tied has a normal tongue at birth; it just seems short to parents who are unaware of a newborn¡¯s
appearance. Tongue tie is often evaluated based on the mobility and how close to the tip of the tongue the
leading edge of the frenulum is attached. Types 1 and 2 are the most common, accounting for 75% of tongue-
ties and are often called classic and anterior ties.Types 3 and 4 account for remaining 25 % and are often
referred as posterior ties.

The Hazelbaker Assessment tool can also be used to evaluate an infant¡¯s tongue-tie. The grading levels range
from Class I (least severe) to Class IV (most severe) and are based on the tongue¡¯s appearance and function
(Hazelbaker, 2010). With the increased emphasis on initiating and maintaining breastfeeding, a resurgence of
feeding difficulties due to tongue-tie has been identified. In addition, decreased tongue mobility may cause
short- and long-term consequences including feeding, speech, orthodontic problems, mandibular abnormalities,
and difficulty with oral cleanliness (Academy of Breastfeeding Medicine [ABM], 2017).

This condition may cause difficulty with breastfeeding due to difficulty with tongue mobility and the ability to
latch to create a strong suction at the breast. Mother’s will often note that their infants (with a tongue-tie) tend
to slip off the breast and cannot maintain a long latch without falling asleep. In addition, this mislatch will often
cause moderate to severe nipple pain in about 80% of breastfeeding mothers. If it does, then a frenotomy release
can be performed in the newborn period, typically by a trained physician, pediatric dentist, or advanced practice
registered nurse (APRN). Ankyloglossia or tongue-tie are associated with a 25% to 60% incidence of
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breastfeeding difficulty and contributes to 10% to 26% of early cessation of breastfeeding (Wong, Patel, Cohen,
et al., 2017). Current literature supports frenotomy for treatment of clinically significant tongue-tie that is
causing difficulty with breastfeeding. Early identification of tongue-tie allows for close evaluation of
breastfeeding and possible difficulties that might arise and referral to a specialist for a frenotomy if indicated.
The majority of infants with tongue-tie have the ability to breastfeed successfully but may require extra
lactation support, guidance, and feeding assistance by additional specialists, such as a lactation consultant
(ABM, 2017).

THYROGLOSSAL CYSTS
A thyroglossal cyst arises from an embryogenic fault that leaves a cyst formed at the base of the tongue, which
then drains through a fistula (an abnormal or surgically made passage between a hollow or tubular organ and the
body surface, or between two hollow or tubular organs) to the anterior surface of the neck (Gowda & Joseph,
2017). Thyroglossal cysts are congenital defects located in or around the midline of the neck, extending to the
base of the tongue. This condition may occur as an autosomal dominant trait. The cyst may involve the hyoid
bone (the bone at the anterior surface of the neck at the root of the tongue) and may contain aberrant thyroid
gland tissue. As the cyst fills with fluid, swelling and obstruction can lead to respiratory difficulty from pressure
on the trachea. If infected, the cyst often appears swollen and reddened, with drainage of mucus or pus from the
anterior neck and requires antibiotic therapy.

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 The cyst is surgically removed to avoid future infection of the space and, if thyroid tissue is present, the
possibility of developing thyroid carcinoma later in life. Observe infants closely in the immediate postoperative
period for respiratory distress because the operative area will develop at least minimum edema from surgical
trauma. Position infants on their sides so secretions drain freely from their mouths. Intravenous (IV) fluid
therapy is given after surgery until the edema at the incision recedes somewhat and swallowing is safe once
more (approximately 24 hours). If the mother is breastfeeding, encourage her to express her milk via a hospital-
grade pump or manual expression during this time to preserve her milk supply. Observe infants closely the first
time they take fluid orally to be certain they do not aspirate. Be certain parents have a chance to feed their infant
before the infant is discharged from the surgical unit so they can be assured the infant is swallowing safely, thus
allowing them to feel confident at feeding the infant at home in a relaxed and comfortable way.

OROFACIAL CLEFTS: CLEFT LIP AND CLEFT PALATE

The maxillary and median nasal processes normally fuse between weeks 5 and 8 of intrauterine life. In infants
with cleft lip, the fusion fails to occur in varying degrees,causing this disorder to range from a small notch in the
upper lip to total separation of the lip and facial structures up into the floor of the nose, with even the upper teeth
and gingiva absent. The deviation may be unilateral or bilateral. The infant’s nose generally appears flattened
because the incomplete fusion of the upper lip has allowed it to expand in a horizontal dimension. Overall rates of
cleft lip and/or palate in the United States average to 1 in 600 newborns (American Cleft Palate & Craniofacial
Association, n.d.).

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Cleft lip is the most common orofacial cleft. It is more prevalent among boys than girls and occurs at a rate of
approximately 20 cases per day and 7,500 cases each year in the United States (Tolarova & Elluru, 2015). This
incidence is highest in the Asian population at 1 out of every 500 people, moderate in the European-derived
population at 1 out of 1,000 people, and significantly lower in the African-derived population at 1 out of 2,500
people. It appears to be caused by the transmission of multiple genes aided by teratogenic factors present during
weeks 5 to 8 of intrauterine life, such as a viral infection, certain seizure medicines such as phenytoin, maternal
smoking or binge drinking, hyperthermia, stress, and maternal obesity (Funato & Nakamura, 2017). Folic acid
deficiency may also be associated with incomplete anterior midline closures (Funato & Nakamura, 2017).

Because of the genetic influence, parents of a child with a cleft lip should be referred for genetic counseling to
ensure they understand they have a small increased chance of having another child with a cleft lip or palate and
that any future children are at a greater risk than usual for this problem.

A cleft palate is an opening of the palate and occurs when the palatal process does not close as usual at
approximately weeks 9 to 12 of intrauterine life. The incomplete closure is usually on the midline and may
involve the anterior hard palate, the posterior soft palate, or both (see Fig. 27.7B). It may occur as a separate
anomaly or in conjunction with a cleft lip. As a single entity, in contrast to cleft lip, it tends to occur more
frequently in girls than boys. Like cleft lip, it appears to be the result of polygenic inheritance or environmental
influences. In connection with cleft lip, the incidence is approximately 1 out of every 1,000 births. As a single
entity, it occurs in approximately 1 out of every 2,000 births. Almost 30% of children with both cleft lip and
palate have associated birth defects, or the cleft palate occurs as only a portion of a larger syndrome (Dixon,
Marazita, Beaty, et al., 2011).

Assessment

Cleft lip may be detected by a sonogram while an infant is in utero. If not detected then, it is readily apparent on
inspection of the mouth at birth. When assessing newborns, be sure you have good lighting so you can visualize
the palate clearly. Because cleft palate is a component of many syndromes, assess the child for other congenital
anomalies as well.

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Therapeutic Management

If a cleft lip is discovered while the infant is still in utero, fetal surgery can repair the condition, although this
procedure is not usually attempted. If the disorder is discovered at birth, a cleft lip can be repaired surgically
shortly thereafter, often at the time of the initial hospital stay or between 2 and 12 weeks of age. Because the
deviation of the lip interferes with sucking, infants may be a better surgical risk as newborns than they are after
a month or more of poor nourishment. Early repair also helps infants experience the pleasure of sucking as soon
as possible. It is equally important from a psychological standpoint as a parent may need caring support to bond
with an infant whose face is deformed in this way (Nidey, Moreno Uribe, Marazita, et al., 2016). Because facial
contours change as a child grows, a revision of the original repair or a nasal rhinoplasty to straighten a deviated
nasal septum may be necessary when the child reaches 4 to 6 years of age. Some infants may have a nasal mold
apparatus applied before surgery to shape a better nostril (Funato & Nakamura, 2017).

The optimal time for repair of a cleft palate is controversial because early repair increases speech development
but may result in a necessary second-stage repair as the child’s palate arch expands with growth (Taub &
Piccolo, 2016). Surgery may be recommended as a two-stage palate repair, with soft palate repair at 3 to 6
months of age and hard palate repair at 6 to 18 months of age, called the Malek protocol. This type of repair
results in less need for future surgery and better facial results (Taub & Piccolo, 2016). Using infant orthodontic
devices and delaying hard palate closure until later has not been shown to increase speech clarity or overall
wellness (Taub & Piccolo, 2016).

Currently, the results of surgical repair of cleft lip and cleft palate are excellent (Fig.27.8). It is helpful to show
parents photographs of babies with good repairs to assure them their child’s outcome can also be as successful.

An infant showing surgical repair of cleft lip. Parents can be encouraged that the results of cleft lip repair are
generally excellent.

One issue that may remain is that because palate repair narrows the upper dental arch, a child may be left with
less space in the upper jaw for the eruption of teeth, creating poor teeth alignment. All children born with a cleft
palate, therefore, need follow-up treatment by a pediatric dentist skilled in children¡¯s dental problems, so that
as the child grows, extractions or realignment of teeth can be done as indicated. Children also need follow-up to
detect if hearing or speech difficulty occurs; because the angle of the eustachian tube may be changed in
surgery, a child may develop more ear infections than usual, possibly leading to some hearing impairment.
After surgical repair, about 80% of children affected by cleft palate progress to develop normal speech, yet
referral to speech therapy early in infancy should always occur to ensure successful speech development
(American Cleft Palate¨CCraniofacial Association, n.d.).

Children with cleft problems tend to receive better, more frequent, and well- coordinated care when seen in an
interprofessional team setting including pediatric dentists, audiologists, speech pathologists, geneticists, and
craniofacial surgeons, so referring parents to an appropriate interprofessional center before discharge is critical
for these infants and their families (Taub & Piccolo, 2016).

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 Nursing Diagnoses and Related Interventions

Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to feeding problem
caused by cleft lip or palate Outcome Evaluation: Newborn ingests an adequate diet of 110 kcal/kg (50 kcal/lb)
in 24 hours; weight is maintained within 10% of birth weight. Preoperative Period. Before a cleft lip or palate is
repaired, feeding the infant becomes a concern because the infant has difficulty maintaining suction with a
bottle or breast; there is evidence of slower growth when compared to infants without a cleft disorder (Taub &
Piccolo, 2016). In addition, it is important the child does not aspirate.

It may be possible for an infant with a cleft lip to breastfeed because the bulk of the mother’s breast tends to
form a seal against the incomplete upper lip. Although the baby needs the enjoyment of sucking, some surgeons
do not want a baby to either breastfeed or suck on a nipple, however, before surgical correction of the disorder
to avoid any local bruising of tissue. Therefore, the best feeding method for the child with cleft lip may be to
support the baby in an upright position and feed the infant gently using a soft bottle and a commercial cleft lip
nipple or a spoon. Breck or Haberman feeders are commercial apparatuses similar to a bulb syringe, which also
can be used (Fig. 27.9). If the surgical repair for a cleft lip will be done immediately, the mother will be able to
breastfeed as early as 7 to 10 days after surgery. Typically after the surgical repair and healing, the baby can
latch to the breast. If there is difficulty with latching, a nipple shield can be suggested to help form a tighter seal
at the breast. Review with the mother how to pump or manually express breast milk to maintain a milk supply
prior to surgical correction and after, if needed. If surgery will be delayed for 1 month, the mother will need
continuing support from the nursing staff to support her efforts on pumping and to remind her that her breast
milk will be very beneficial to her infant and the healing process.

Figure 27.9 (A) Specialty feeding devices used for infants with cleft
lip and cleft palate. (B) An infant uses a Haberman Feeder.

If a cleft extends to the nares so the nose and mouth are joined, breathing causes the oral mucous membranes
and lips to become dry. Offering small sips of fluid between feedings can help keep the mucous membranes
moist and prevent cracks and fissures that could lead to infection. Following a feeding, be certain an infant with
a cleft lip is burped well because the inability to grasp a nipple or syringe edge securely causes the infant to
swallow more air than usual.

Infants with a cleft palate cannot suck effectively either because pressing their tongue or a nipple against the
roof of their mouth forces milk up into their pharynx, possibly leading to aspiration. The most successful
method for feeding this infant, like the child with a cleft lip, therefore, is to use a commercial cleft palate nipple
that has an extra flange of rubber to close the roof of the mouth. If the nipple is used with a plastic bottle that
can be squeezed gently to increase the flow of the feeding, this can compensate for poor sucking. A Breck
feeder may also be used to feed infants with a cleft palate.

If surgery will be delayed beyond 6 months of age or the time when solid food would usually be introduced,
teach parents to be certain any food they offer is soft because particles of coarse food could invade the
nasopharynx and be a cause of aspiration.

Postoperative Period. After surgery for cleft lip or palate, an infant is kept nothing by mouth (NPO) for
approximately 4 hours and then introduced to liquids such as plain water. Be certain to begin this process with
only a small amount each time to prevent vomiting.

It’s important that no tension is placed on a lip suture line because this helps keep the sutures from pulling apart
and leaving a large scar. During this immediate postoperative period, therefore, the infant is usually fed using a
specialized feeder because this causes less suture line tension than bottle feeding or breastfeeding.

After palate surgery, only liquids are generally given the first 3 or 4 days, and then a soft diet is followed until
healing is complete. Ask parents before surgery what fluids the child prefers so they can be made available
postsurgery.

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When children begin to eat soft food, observe they don’t use a spoon because spoons can invariably be pushed
against the roof of their mouth and possibly disrupt sutures. If being fed rather than allowing the infant to use a
spoon evokes an intense reaction, it is probably better to leave a child on a liquid diet until the sutures are
removed. Be certain milk is not included in the first fluids offered because milk curds tend to adhere to the
suture line and so are difficult to remove. After a feeding, always offer the child a sip of clear water to rinse the
suture line and keep it as clean as possible. Also, educate parents to be diligent about oral health care. In infants
with clefts involving the maxillary alveolar ridge (upper gum), it is common for some teeth typically the upper
incisors and cuspids on the side of the cleft to be misshapen or turned (Cleft Palate Foundation, 2010). Prudent
twice-daily gum and teeth brushing with an age appropriate toothbrush and tooth paste is crucial, as are by early
dental visits for monitoring.

Nursing Diagnosis: Risk for ineffective airway clearance related to oral surgery

Outcome Evaluation: Child’s respiratory rate remains at baseline level for age without retractions or obvious
distress.

Because of the local edema that occurs after cleft lip or palate surgery, it’s important to observe children closely
in the immediate postoperative period for respiratory distress. Remember that before surgery, the infant with a
cleft lip may have breathed through the mouth. After surgery, the infant now has to learn to breathe through the
nose, possibly adding to respiratory difficulty. Generally, however, this is not a problem because newborns
normally are strict nose breathers.

Following either cleft lip or cleft palate surgery, infants may need their mouth suctioned to remove mucus,
blood, and unswallowed saliva. When doing this, be exceedingly gentle so you don¡¯t touch the suture line with
the catheter. Place infants on their side to allow mouth secretions to drain forward. And, following a cleft lip
repair, be sure a child does not turn onto his or her abdomen because this could put pressure on the suture line,
possibly tearing it. Placing the child in an infant bouncy chair is another possibility.

Nursing Diagnosis: Impaired tissue integrity at incision line related to cleft lip or cleft palate surgery

Outcome Evaluation: Incision line appears clean and intact and free of erythema or drainage during
postoperative period.

After cleft lip surgery, the suture line may be held in close approximation by a Logan bar (a wire bow taped to
both cheeks; Fig. 27.10) or an adhesive bandage such as a Band-Aid simulating a bar that brings together the
incision line but does not cover the incision. Assess the simulated or Logan bar is secure and continues to
protect the suture line from tension after each feeding or cleaning of the suture line. Furnish adequate pain relief
also so the infant does not cry because this puts increased tension on the sutures. To help avoid crying, try to
anticipate the infant’s needs by having formula ready to feed. Help the parents use whatever measures, such as
rocking, carrying, or holding, that are necessary to make the infant feel secure and comfortable.

A Logan bar is an apparatus that may be used to protect the surgical incision for a cleft lip repair.

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Nothing hard or sharp must come in contact with a recent cleft suture line. Observe infants after palate repair
carefully, therefore, to be certain they do not put toys with sharp edges into their mouths. It¡¯s also good
practice to not allow them to use a straw to drink or hold a toothbrush to clean their teeth so they don¡¯t brush
the suture line accidentally. Keep elbow restraints in place as necessary so they do not put their fingers in their
mouth and poke or pull at the sutures. Sutures on the lip or palate feel extremely odd, so most children not only
run their tongue over their sutures but also don’t respond to advice not to do this. Because this often occurs
when children have nothing to think about, help the parents provide diversional activities such as reading or
singing to keep the child’s attention off the suture line.

If parents will be continuing to give an analgesic such as acetaminophen (Tylenol) or ibuprofen (Advil) after
they return home, be certain they are aware of the correct dosage and time schedule for administration of the
liquid forms. They must also continue to keep the suture line protected until healing is complete.

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Nursing Diagnosis: Risk for infection related to surgical incision

Outcome Evaluation: Infant’s temperature is below 98.6F (37C) by tympanic membrane; incision site is clean,
dry, and intact without erythema or foul drainage. An infection and subsequent scarring may result if crusts
from serous drainage are allowed to form on a cleft lip suture line. Most surgeons, therefore, prescribe cleaning
the suture line with sterile water or sterile saline with sterile cotton-tipped applicators after every feeding or
whenever the normal serum that forms on suture lines accumulates. Do not rub the suture line; use a smooth,
gentle, rolling motion to avoid loosening sutures. Gently dry the suture line with a dry sterile cotton-tipped
applicator afterward. Remember that the infant has sutures on the inside of the lip that need the same meticulous
care as those visible on the outside.

Nursing Diagnosis: Risk for impaired parenting related to birth of an infant who is physically challenged

Outcome Evaluation: Parents state they believe there will be a positive outcome for their child and demonstrate
positive coping behaviors evidenced by holding and helping with infant care.

To promote effective bonding, parents need to hold and interact with their infant during both the preoperative
and postoperative periods. Caution them the child’s incision line will appear swollen in the immediate
postoperative period, but its appearance will improve with time. As soon as the child’s sutures have been
removed, parents may breastfeed or feed with an ordinary bottle. Caution both the breastfeeding mother (who
has been maintaining her milk supply through expression) and the formula-feeding mother that because the
infant has never sucked before, time will be needed to learn how to suck, just like a newborn.

Observe whether parents look at their baby’s face while feeding the baby. Help them to understand that any
negative feelings they feel toward the child or themselves, such as sadness or anger, are normal. This assurance
does not instantly make them feel better about what has happened, but the knowledge the feelings they are
experiencing are normal can help them begin to deal with such emotions. Many communities have support
groups for parents of children born with a cleft lip or palate. Referral to these groups can offer parents
additional support. The National Cleft Palate Foundation provides parent education materials at
www.cleftline.org.

Nursing Diagnosis: Risk for situational low self-esteem related to child’s facial surgery

Outcome Evaluation: Child participates in normal childhood activities that involve contact with other people,
states activities he or she enjoys at healthcare visits, and demonstrates age-appropriate developmental
milestones.

If a scar remains after cleft lip surgery, children may need some help adjusting to their appearance until a
second cosmetic repair can be completed later in life. Reinforce children’s positive attributes, stressing that the
scar is only one small aspect of who they are. As children reach adolescence, you may need to review a familial
inheritance pattern of cleft lip so they are aware of the possible risk for transmission to their own children.

Nursing Diagnosis: Risk for infection (ear) related to the altered angle of the eustachian tube with cleft palate
surgery

Outcome Evaluation: Outcome Evaluation: Parents state signs and symptoms of ear infection and importance of
early treatment; parents list signs of diminished hearing and appropriate agencies for support and guidance.

Changing the contour of the palate when it is repaired also changes the slope of the eustachian tube to the
middle ear. This can lead to a high incidence of middle ear infection (otitis media) because organisms are able
to reach this area from the oral cavity more readily than usual. Review the signs of infection such as fever, pain,
pulling on an ear, or discharge from the ear with parents. Remind them of the importance of reporting
pharyngeal infection to their primary care provider promptly so it can be treated before the infection spreads to
the middle ear. Because the eustachian tube may remain partially closed in its changed position, serous otitis
media (accumulation of fluid in the middle ear) also tends to occur more frequently in these children than in
others. If this happens, myringotomy tubes may be inserted to drain middle ear fluid and to help protect hearing
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(see Chapter 50). Be certain parents understand the need for routine screening for hearing loss during childhood
because this is a common early sign of serous otitis media.

Nursing Diagnosis: Risk for impaired verbal communication related to cleft palate

Outcome Evaluation: Family members voice satisfaction with child’s speech; developmental milestone of
clearly articulated two-word sentences by age 2 years is met.

Infants with a cleft palate will begin to make speech sounds at the normal time (age 2 months). This speech,
however, is usually guttural and unclear as many alphabet sounds are made by touching the tongue against the
roof of the mouth. Depending on the age of the cleft palate repair, some children will continue to have
accompanying speech difficulty because the soft palate must function for the child to pronounce p and b sounds
(Prathanee, Pumnum, Seepuaham, et al., 2016). If cleft palate surgery is going to be delayed past age 2 years (as
might happen if the child has other congenital anomalies, such as heart disease), a plastic prosthesis to cover the
incomplete palate may be prescribed because this allows the child to articulate more efficiency.

Altered speech patterns in children generally do not resolve spontaneously and require intervention by a speech
pathologist prior to school age.

THE PIERRE ROBIN SEQUENCE

The Pierre Robin sequence (also called Pierre Robin syndrome) is a rare triad of micrognathia (small mandible),
cleft palate, and glossoptosis (a tongue malpositioned downward). This triad of conditions can result in severe
upper airway obstruction, which then may cause secondary respiratory distress due to upper airway obstruction.
Estimates of occurrence range from 1 in every 8,500 births to 1 in every 20,000 births; the primary event that
leads to the syndrome tends to be unknown. More than half of the children born with Pierre Robin syndrome
have associated disorders of congenital glaucoma; cataracts; cardiac disorders; or other genetic, chromosomal,
or medical concerns. They need thorough physical and genetic assessments to be certain that none of these other
associated disorders are present (B¨¹tow, Zwahlen, Morkel, et al., 2016).

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Monitor infants for possible airway obstruction (as a result of their small jaw, their tongue tends to drop
backward and obstruct their airway). Suctioning to remove secretions may be necessary. A side-lying rather
than a supine sleeping position is recommended for all newborns and infants (until they can roll over) to avoid
obstruction. Occasionally, infants have such extensive airway obstruction that a suture is attached to the anterior
aspect of the tongue and the mucous membrane of the lower lip to pull the tongue forward and prevent the rear
of the tongue from blocking the upper airway. Maintaining positioning as the infant grows and gains mobility
may be increasingly difficult, but sleep studies have shown these children have severe sleep apnea, with as
many as 49 episodes of apnea leading to oxygen desaturation per hour if sleeping supine (B¨¹tow, Naidoo,
Zwahlen, et al., 2016). Nasopharyngeal airways (NPAs) have been shown to be effective in upper airway
obstruction, and 86.5% of infants studied maintained adequate airways with positioning or temporary placement
of an NPA for several months. The airways of older children can be well maintained using nasal continuous
positive airway pressure (CPAP); with this, after several years of therapy, the episodes of obstructive sleep
apnea decrease to about five per hour, possibly decreasing the need for more invasive surgery.

As the child grows older, the jaw will grow somewhat, although the mandible will always be small. Parents
need instructions on how to feed these infants until the cleft palate can be repaired. The child may have a
gastrostomy tube or button inserted to relieve feeding difficulty (see Chapter 37).

Parents of the child with this syndrome take on a great deal of responsibility when they assume their infant’s
care; it’s best if they are referred to an interprofessional team prior to discharge from the hospital. Be certain
they have the name and number of a primary healthcare provider they can call when they have questions. Many
of these parents grow exhausted during the first few weeks of their child’s life, afraid they may sleep so soundly
at night they miss their child having respiratory difficulty. Using a respiratory monitor at night can be helpful.
As parent’s confidence grows in their ability to provide care, this problem lessens, but it may be months or even
years before a high level of confidence is achieved.

ESOPHAGEAL ATRESIAS AND TRACHEOESOPHAGEAL

FISTULAS
Between weeks 4 and 8 of intrauterine life, the laryngotracheal groove in the chest develops into the larynx,
trachea, and beginning lung tissue; the esophageal lumen forms parallel to this. A number of anomalies can

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 occur if the trachea and esophagus are affected by some teratogen that does not allow the two organs to fully
form separately and possibly become connected.

Esophageal atresia is the incomplete formation of the esophageal lumen, resulting in the proximal (upper)
esophagus forming a ¡°blind pouch,¡± which then does not connect to any other structure. Tracheoesophageal
fistula occurs when an opening develops between the closed distal (lower) esophagus and the trachea. Five
usual types of esophageal atresia that result are:

1. The esophagus ends in a blind pouch; there is a tracheoesophageal fistula between the distal part of the
esophagus and the trachea (Fig. 27.11A).

2. The esophagus ends in a blind pouch; there is no connection to the trachea (see Fig. 27.11B).

3. A fistula is present between an otherwise normal esophagus and trachea (see Fig. 27.11C).

4. The esophagus ends in a blind pouch. A fistula connects the blind pouch of the proximal esophagus to the
trachea (see Fig. 27.11D).

5. There is a blind end portion of the esophagus. Fistulas are present between both widely spaced segments o
the esophagus and the trachea (see Fig. 27.11E).

Figure 27.11 An esophageal atresia and a tracheoesophageal fistula. (A) In the most common type of
esophageal atresia, the esophagus ends in a blind pouch. The trachea communicates by a fistula with the lower
esophagus and stomach (approximately 90% of infants with the defect have this type). (B) Both upper and
lower segments end in blind pouches (5% to 8% of infants with the defect have this type). (C) Both upper and
lower segments communicate with the trachea (2% to 3% of infants with the defect have this type). (D) Very
rarely, the upper segment ends in a blind pouch and communicates by a fistula to the trachea, or (E) a fistula
connects to both upper and lower segments of the esophagus.

All of these are potentially serious disorders because, during a feeding, milk can fill the blind pouch of the
upper esophagus and then overflow into the trachea, or a fistula can allow milk to enter the trachea from the
esophagus, resulting in aspiration. Even if not fed, the swallowed saliva of the baby will fill the esophagus and
overflow into the airway, resulting in repeated aspiration. The incidence of the disorder ranges from 1 out of
2,500 to 4,500 live births (Vergouwe, IJsselstijn, Wijnen, et al., 2015).

Assessment

Esophageal atresia must be ruled out in any infant born to a woman with hydramnios (excessive amniotic fluid).
This is because hydramnios occurs because, normally, a fetus swallows amniotic fluid during intrauterine life. A
fetus with esophageal atresia cannot effectively swallow, so the amount of amniotic fluid can grow abnormally
large. Many infants with esophageal atresia are born preterm because of the accompanying hydramnios,
compounding their original problem with immaturity. The infant needs to be examined carefully for other
congenital anomalies that could have occurred from the teratogenic effect at the same week in gestation, such as
vertebral, anal, cardiac, tracheoesophageal, renal, and limb anomalies (VACTERL syndrome) (Vergouwe et al.,
2015).

If not diagnosed in utero, diagnosing a child who has an esophageal atresia/tracheoesophageal fistula before the
infant is first fed is important. Otherwise, the infant will cough, become cyanotic, and have obvious difficulty
breathing as fluid is aspirated. Newborns who have so much mucus in their mouths that they appear to be
blowing bubbles should be suspected of having either disorder. Esophageal atresia can be diagnosed with
certainty if a catheter cannot be passed through the infant’s esophagus to the stomach so stomach contents can
be aspirated. If helping with this, supply a firm catheter because a soft one will curl in a blind-end esophagus
and appear to have passed. An X-ray will reveal a radiopaque catheter coiled in the blind end of the esophagus.
A flat-plate X-ray of the abdomen or an ultrasound also may reveal a stomach distended with the air that has
passed from the trachea into the esophagus and stomach, demonstrating that a fistula is present. Either a barium
swallow or a bronchial endoscopy examination can also reveal the blind-end esophagus and fistula.
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Therapeutic Management

Emergency actions are required for the infant with this group of esophageal disorders in order to prevent the
development of pneumonia, one of the major complications that is apt to occur from leakage of oral or stomach
secretions into the lungs. The best position for the infant is an upright, 60-degree angle so acid stomach fluids
stay in the stomach through the use of gravity (sitting the baby in an infant chair does this). If a nasogastric tube
is inserted into the distal end of the esophagus and connected to low intermittent suction, oral secretions will no
longer collect in the blind pouch, decreasing aspiration risks. A gastrostomy may be inserted into the stomach
(under local anesthesia) with the tube allowed to drain by gravity as another way of keeping the stomach empty
of secretions and preventing reflux into the lungs. IV fluids and parenteral nutrition are begun to prevent
dehydration and an electrolyte imbalance from lack of oral intake. IV medications that restrict the stomach’s
ability to produce acid may be prescribed, and an antibiotic may be prescribed to help prevent an infection.

When the infant has been stabilized by this sequence of measure, surgery consists of closing the fistula and
anastomosing the esophageal segments. If the defect was only a simple fistula, surgery may be done by
thoracoscopy to minimize the time required for surgery (Vergouwe et al., 2015). If the repair will be more
complex, it may be necessary for the surgery to be completed in different stages and to use a portion of the
intestine to complete the anastomosis if the esophageal segments are too far apart to join readily. After an
anastomosis repair, observe infants closely at postoperative days 7 to 10 because this is the point when their
internal sutures will dissolve, possibly allowing a leak from the anastomosis site to occur at this time. If such a
leak occurs, fluid and air can leak out into the chest cavity, and pneumothorax (collapse of a lung) can occur.

In some infants, some stenosis or stricture at the anastomosis site remains. If this occurs, esophageal dilatation
at periodic intervals to keep the repaired esophagus fully patent may be necessary. Gastroesophageal reflux
may also occur after a repair, especially if the esophagus is left shorter than usual; this can lead to recurrent
fistula formation and irritation from the presence of stomach acid in the esophagus (Vergouwe et al., 2015).

The ultimate prognosis for children with this disorder depends on the extent of the repair necessary, the
condition of the child at the time of surgery, and the presence or absence of other congenital anomalies. Even
with larger disorders, if surgery can be performed before pneumonia develops, the repair of the esophagus and
trachea will be effective. The mortality rate for the condition remains high, however, because of the presence of
other congenital disorders and low birth weight that often accompanies the tracheal abnormality.

Nursing Diagnoses and Related Interventions

Both nursing diagnosis and outcomes established for the child with either a tracheoesophageal fistula or
esophageal atresia must be realistic in terms of the extent of the disorder, the timing of anticipated surgery, and
the stage of grief or readiness for decision making and planning the parents have reached.

Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to inability to take in
oral feedings

Outcome Evaluation: Child maintains weight within 10% of birth weight and maintains weight in same
percentile on growth curve.

Before surgery, because oral fluid cannot be given until the esophagus is repaired, IV therapy or total parenteral
nutrition (TPN) can be used to supply fluid and calories. Following surgery, the infant may be fed orally, may
be continued on TPN, or may be started on gastrostomy feedings, depending on whether the surgery could be
completed in one stage or not.

Early introduction of oral fluid may help to ensure patency of the esophagus because it helps to decrease
adhesion formation at the anastomosis site and also allows the infant the enjoyment and practice of sucking. If
formula is given by gastrostomy feedings, be certain to introduce it into the tube slowly and allow it to run by
gravity pressure only to prevent fluid from entering the esophagus and putting pressure on the new suture line.
After the feeding, the end of the tube should be elevated, covered by sterile gauze, and kept in the elevated
position, not clamped, so any air introduced during the feeding will be released from the tube and not enter the
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esophagus to put pressure on the suture line. This also helps to ensure that if the infant should vomit the feeding,
the vomitus will be projected into the gastrostomy tube and again will not contaminate the fresh sutures. Most
newborns enjoy sucking a pacifier during gastrostomy feedings for sucking pleasure. If a mother wishes to
breastfeed, she can manually express breast milk for the gastrostomy feedings.

If the child is to return home to await a second-stage operation, the gastrostomy tube will be left in place. Be
certain parents are comfortable doing gastrostomy feedings as well as usual infant care, such as holding or
talking to the infant in the face of this different feeding method.

Nursing Diagnosis: Risk for infection related to aspiration or seepage of stomach secretions into lungs

Outcome Evaluation: Child’s temperature remains below 98.6F (37C) axillary; there is absence of rales on
auscultation.

Preoperative Care. In addition to measures such as keeping the child in a sitting position, if surgery will be
significantly delayed, an infant may have a cervical esophagostomy (the distal end of the blind esophagus is
brought to the surface just over the sternum) as a method to allow oral secretions to drain. Use absorbent gauze
around the opening of the site to absorb moisture and apply a protective ointment liberally to the skin to help
prevent excoriation of the skin. Consult as necessary with a wound, ostomy, and continence therapy nurse to
prevent further skin irritation.

Keeping the infant under a radiant heat warmer with a high-humidity oxygen source helps to maintain body heat
and liquefy bronchial secretions. Try to keep the infant from crying because, with crying, air enters the stomach
from the trachea, distending the stomach and possibly causing vomiting with aspiration into the lungs. A
pacifier may be helpful to relax the baby and also to satisfy a sucking need.

Postoperative Care. The baby will return from final surgery with multiple tubes (e.g., chest tubes, endotracheal
tube, Foley catheter, multiple IV lines, probably an arterial line, possibly a gastrostomy tube if not previously
inserted), as well as blood pressure, oxygen saturation, and electrocardiography monitoring equipment. The
posterior chest tube drains collecting fluid, and the anterior tube allows air to leave the chest space so the lungs
can re-expand (see Chapter 41 for care of an infant with chest tubes in place).

Anticipatory teaching about how the infant will appear is important for the family at this stressful time to keep
them from growing overwhelmed. Skilled nursing care of both the child and family is essential.

In the first few days after surgery, a major assessment is to observe the infant for respiratory distress. Continue
to suction saliva from the mouth as prescribed because this tends to continue to accumulate in the pharynx.
Suctioning must be done only shallowly, however, to prevent the suction catheter from touching the suture line
in the esophagus. Turning the child frequently discourages fluid from accumulating in the lungs. Humidified
oxygen helps to keep respiratory secretions moist and not become stagnant. Keep an infant laryngoscope and
endotracheal tube readily available at the bedside in case extreme edema develops, which may increase the
infant’s risk for airway obstruction.

Nursing Diagnosis: Risk for impaired skin integrity related to gastrostomy tube insertion site

Outcome Evaluation: Skin surrounding gastrostomy tube remains clean, dry, and without erythema.

Gastric secretions, which are highly acidic, can leak onto the skin from the gastrostomy site, leading to skin
irritation. Protect the skin by using a cream or commercial skin protection system. If the problem is not easily
resolved, consult with a wound, ostomy, and continence therapy nurse to reduce the possibility of skin irritation
and infection.

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 ABDOMINAL WALL DEFECTS
Abdominal wall defects, with the exception of umbilical hernia, are potentially serious disorders because they
directly affect the gastrointestinal system and infant nutrition.

1. Umbilical Hernias
An umbilical hernia is a protrusion of a portion of the intestine through the umbilical ring, muscle, and fascia
surrounding the umbilical cord (Mirza & Ali, 2016). This creates a bulging protrusion under the skin at the
umbilicus. It is rarely noticeable at birth while the cord is still present but becomes increasingly noticeable at
healthcare visits during the first year.

Umbilical hernias occur most frequently in Black children (seen in 30% term infants), low¨Cbirth-weight
infants, and more often in girls than in boys. The structure is generally 1 to 2 cm (0.5 to 1 in.) in diameter but
may be as large as an orange when children cry or strain. The size of the protruding mass is not as important as
the size of the fascial ring through which the intestine protrudes. If this fascial ring is less than 2 cm, closure
will usually occur spontaneously after the child begins to walk to around 2 years of age, so surgical repair is
often not necessary for mild (small) cases. If the fascial ring is larger than 2 cm, ambulatory surgery repair is
generally indicated to prevent herniation and intestinal obstruction or bowel strangulation. This is usually done
at 1 to 2 years of age. If the small umbilical hernia has not closed by preschool age, a repair will often be done
when the child is 4 to 5 years old.

Some parents believe holding an umbilical hernia in place by using belly bands or taping a silver dollar over the
area will help reduce the hernia. These actions can actually lead to bowel strangulation and so should be
avoided.

The child returns from surgery with a dressing, which remains in place until the sutures are well healed. Remind
parents to sponge bathe the child until they return for a postoperative visit when the dressing is removed. If the
child is not yet toilet trained, caution parents to keep diapers folded well below the dressing to prevent
contaminating the suture line with stool.

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 2. Omphaloceles
An omphalocele is a protrusion (herniation) of abdominal contents through the abdominal wall at the point of
the junction of the umbilical cord and abdomen (Fig. 27.12). The herniated organs involved are usually the
intestines, but they may include the stomach and liver. Occurring about 1 out of 5,000 live births, the organs are
usually covered and contained by a thin transparent layer of amnion and chorion with the umbilical cord
protruding from the exposed sac. This condition occurs because, at approximately weeks 6 to 8 of intrauterine
life, the fetal abdominal contents, which grow faster than the fetal abdomen, are pushed out from the abdomen
into the base of the umbilical cord. At 7 to 10 weeks, when the fetal abdomen has enlarged sufficiently, the
intestine normally returns to the abdomen. An omphalocele occurs when abdominal contents fail to return in the
usual way. In 67% of cases, the occurrence is associated with other congenital disorders such as cardiac,
neurologic, genitourinary, skeletal, and chromosomal abnormalities (Tappero & Honeyfield, 2015).

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 3. Gastroschisis

Gastroschisis, a term derived from the Greek word for stomach cleft or fissure, is a condition similar to an
omphalocele, except the abdominal wall disorder is a distance from the umbilicus, usually to the right, and
abdominal organs are not contained by a membrane but rather spill freely from the abdominal wall (Jones,
Isenburg, Salemi, et al., 2016). Also, a greater amount of intestinal contents tends to herniate, increasing the
potential for volvulus and obstruction. The condition occurs because of failure of the abdominal wall to close,
usually during the fourth week of development, and its incidence is about 4 to 5 per 10,000 live births. The
disorder is similar to a neural tube defect (covered later in this chapter) because both are a failure to close at
about the same gestational time. Children with gastroschisis often have decreased bowel motility and, even after
surgical correction, may have difficulty with absorption of nutrients and passage of stool. Long-term follow-up
may be necessary to ensure that nutrition and elimination are adequate (Jones et al., 2016).

Assessment

The incidence of omphalocele remains steady, whereas the incidence of gastroschisis is steadily rising, perhaps
associated with maternal obesity (Jones et al., 2016). The wall defect with the herniated organs may be
identified by sonogram during intrauterine life (Jones et al., 2016). These may also be revealed by an elevated
maternal serum ¦Á- fetoprotein (MAFP) examination during pregnancy, which is done at the 15th week of
pregnancy; the level of MAFP will be abnormally increased if there is an open spinal or abdominal lesion. If the
result is elevated, an amniocentesis is then done to assess the level of AFP in amniotic fluid. A prenatal
sonogram is also helpful to determine the presence of both abdominal wall or spinal disorders (see Chapter 11
for further discussion of these prenatal assessments). If an omphalocele or gastroschisis is not identified during
pregnancy, their presence is obvious on inspection at birth. When an omphalocele or gastroschisis is identified
in utero, a cesarean birth may be performed to protect the exposed intestine. If this is the only disorder
identified, however, a vaginal birth may be preferred. Be certain to document the general appearance of the
defect and its size in centimeters at birth to serve as a baseline assessment. Both conditions can vary greatly in
size, from small, where the exposed intestine is the size of a ping-pong ball, to extensive, where all of the
abdominal contents are externalized.

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Therapeutic Management

With both omphalocele and gastroschisis, until surgery and the bowel is effectively returned to the abdomen,
the infant will be fed by TPN to supply nutrients and keep the bowel from filling with air or stool. Most infants
with gastroschisis will have surgery within 24 hours to replace the bowel before the blood supply becomes
hampered, the intestinal membranes dry, bowel volvulus occurs, or the bowel becomes infected. It is often
difficult to replace the entire bowel with immediate surgery because the infant’s abdomen, which did not need
to grow to accommodate the abdominal contents, is smaller at birth than usual. Replacing the total bowel into
this small abdomen could result in respiratory distress from the pressure of the visceral bulk on the diaphragm
and lungs. Also, the bowel might not have room for effective peristalsis. If a gastroschisis is small, a one-stage
repair may be possible. If large, one surgical approach is the use of a prosthetic patch repair that bridges the
unformed gap on the abdomen with a synthetic material; the skin is then drawn tight and closed over the patch.
A second approach is to replace only a portion of the bowel at one time. The remainder is contained by a
Silastic pouch termed a silo that is suspended over the infant¡¯s bed. Over the next 5 to 7 days, the bowel is
gradually returned to the abdomen by multiple surgical procedures (O¡¯Connell, Dotters-Katz, Kuller, et al.,
2016).

For an omphalocele, if the sac is ruptured, the defect is treated like gastroschisis because of the potential for
infection. If unruptured, an external dressing producing mild pressure may be used over the intact membrane.
This gradually compresses the abdominal contents, allows the skin to stretch between treatments, and does not
appear to be a painful procedure.

Nursing Diagnoses and Related Interventions

Both nursing diagnosis and outcomes established for the infant with gastroschisis or an omphalocele, like those
with esophageal disorders, must be realistic in terms of the extent of the disorder, the timing of anticipated
surgery, and the stage of grief or readiness for decision making and planning the parents have reached.
Gastroschisis and omphalocele defects can be an extreme shock to parents because they are conditions that are
obviously severe yet also generally unknown.

Nursing Diagnosis: Risk for infection related to exposed abdominal contents

Outcome Evaluation: Child’s temperature remains below 98.6F (37C) axillary; skin surrounding omphalocele
remains clean, dry, and intact, without erythema or foul drainage.

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Before surgery, it is important that the lining of the peritoneum covering an omphalocele not be ruptured or
allowed to dry and crack; if this happens, infection and malrotation of the uncontained intestine can then occur,
thus complicating the surgical repair. Exposure of the intestine to air also causes a rapid loss of body
heat.Therefore, be certain not to leave an infant with either gastroschisis or an omphalocele under a radiant heat
source in the birthing room because this will quickly dry the exposed bowel. Place the baby in a warmed
incubator instead. In both instances, also cover the herniated bowel with either sterile, warm, saline-soaked
gauze or a sterile plastic bowel bag until surgery can be scheduled. Because of the large amount of exposed
intestinal surface, be certain the saline is at body temperature to prevent lowering the baby¡¯s body temperature.

The prognosis for a final successful surgical repair with both these conditions is good (Emil, Canvasser, Chen,
et al., 2012). Except for a large abdominal scar, following surgery, the child who had gastroschisis or an
omphalocele will be the child originally envisioned by parents. If the size of the scar is a problem for the child
in later life, cosmetic surgery can reduce its appearance.

Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to exposed abdominal
contents

Outcome Evaluation: Child’s weight remains within 10% of birth weight; skin turgor is good; specific gravity
of urine is 1.003 to 1.030.

Infants are maintained on TPN prior to surgery. A nasogastric tube for decompression is inserted at birth to
prevent intestinal distention, which would enlarge the bowel lumen and make it even more difficult to replace.
Do not feed the infant orally or allow the infant to suck on a pacifier until the bowel repair is complete because
doing so would distend the exposed bowel with food or air and would also make its return to the abdomen more
difficult. Some infants have an accompanying volvulus (a twisting of the bowel causing obstruction), which is
another reason to omit oral feedings. After surgery, the infant continues to be maintained on TPN until the final
stage of bowel repair is complete, and breastfeedings or formula feedings can be gradually introduced. Assess
infants carefully for signs of obstruction such as abdominal distention, constipation, diarrhea, or vomiting when
they begin oral feedings to be certain a bowel obstruction or volvulus is not present.

Infants with extensive repairs can be hospitalized for as long as 1 or 2 months until second-stage or even a third-
stage operation is complete (an average is about 30 days). Encourage parents to room-in or visit frequently
because this is a critical adjustment period for both the infant and parents. Be sure the infant has age-
appropriate toys available for stimulation.
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Parents may voice frustration because their child’s treatment is being done in such small stages. Offer support to
help them accept this treatment method as the best way to manage this type of intestinal disorder and to protect
the safety and long-term function of the bowel.

Intestinal Obstructions
If canalization of the intestine does not occur in utero at any point, an atresia (complete closure) or stenosis
(narrowing) of the fetal bowel can develop, although the most common site is the duodenum.

Obstruction may also occur because the mesentery of the bowel twisted as the bowel reentered the abdomen
after being contained in the base of the umbilical cord early in intrauterine life or from looseness of the intestine
in the abdomen after it was returned (Juang & Snyder, 2012). This twisting pattern is termed a volvulus and
continues to be a potential problem for the first 6 months of life until the infant develops firmer intestinal
supports. Yet another reason obstruction can occur is because of thicker-than-usual meconium formation,
blocking the lumen (meconium plug or meconium ileus).

Assessment

Intestinal obstruction may be anticipated if the mother had hydramnios during pregnancy (i.e., swallowed
amniotic fluid could not be absorbed effectively by the fetus) or if more than 30 ml of stomach contents can be
aspirated from the newborn stomach by catheter and syringe at birth (fluid is not passing freely through the
tract). If the obstruction is not revealed by either of these findings, then symptoms of intestinal obstruction in
the neonate are the same as at any other time in life: The infant passes no meconium or may pass one stool
(meconium that formed below the obstruction) and then not pass any more; the abdomen becomes distended
and tender. As the effect of the obstruction progresses, the infant will vomit. Remember, many neonates spit up
feedings when burped. This rapid ejection of milk smells barely sour. True vomiting from intestinal obstruction
is usually sour smelling (stomach acid has acted on it), includes bile, and occurs spontaneously without
coughing or back patting. Vomitus may also be black from the color of meconium.

Bowel sounds will begin to increase in number as the bowel increases peristaltic action in an effort to push stool
past the point of obstruction. Waves of peristalsis may be observable across the abdomen. The infant may reveal
that abdominal pain is developing by crying hard, forceful, indignant crying and by pulling the legs up against
the abdomen. Lastly, the child’s respiratory rate will increase as the intestine fills, the diaphragm is pushed up
harder and harder against the lungs, and lung capacity decreases. An abdominal X-ray, sonogram, MRI, or
barium enema will reveal no air below the level of obstruction in the intestine or isolate the level of the
obstruction.

Therapeutic Management

As soon as a bowel obstruction is confirmed, an orogastric or nasogastric tube is inserted and then attached to
low suction or left open to the air to prevent further gastrointestinal distention (see Chapter 37). Always use low
intermittent suction with decompression tubes in neonates because pressure greater than this can irritate and
ulcerate their sensitive stomach lining.

IV therapy is begun to restore fluid and electrolyte balance; immediate surgery is scheduled to relieve the
obstruction before pressure on the bowel causes death of the involved intestinal lining (Sundaram, Hoffenberg,
Kramer, et al., 2012).

Repair of the obstruction (with the exception of meconium plug syndrome) can usually be accomplished by
laparoscopy, although full abdominal surgery may be necessary. The area of stenosis or atresia is removed, and
the bowel is anastomosed. If the repair is anatomically difficult or the infant has other anomalies that interfere
with overall health, a temporary colostomy may have to be constructed, and the infant discharged to home care
with follow-up surgery rescheduled at 3 to 6 months of age (see Chapter 37 for care of a child with a

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colostomy). If a large portion of the bowel has to be removed, this can have an impact on nutrient absorption
(called short bowel syndrome) as the child grows older.

Nursing Diagnoses and Related Interventions

Nursing Diagnosis: Risk for deficient fluid volume related to vomiting

Outcome Evaluation: Child’s skin turgor is good; capillary refill is 1 to 2 seconds; pulse rate is at least 100 to
120 beats/min; no further vomiting occurs; urine output is at least 1 to 2 ml/kg/hour.

Once an obstruction is suspected, keep an infant NPO to prevent the bowel from filling and causing vomiting
and aspiration. Vomiting is always serious in neonates not only because aspiration may occur but also because
infants can become dehydrated rapidly. They also lose chloride (a component of the hydrochloric acid found in
stomach contents), which then leads to metabolic alkalosis. As a way of compensating for the loss of chloride,
the baby’s kidneys excrete potassium, which can cause infants to become hypokalemic. Keeping an infant NPO,
restoring fluid by IV therapy, and monitoring laboratory values for electrolyte balance until surgery, therefore,
are crucial actions to prevent all these events. Following surgery, TPN or IV fluid will be continued until bowel
sounds return; after a short postoperative course, assuming no other bowel condition such as short bowel
syndrome is present, an infant should have no further problems.

Meconium Plug Syndrome

A meconium plug is an extremely hard portion of meconium that has completely blocked the intestinal lumen,
causing bowel obstruction. The cause is unknown but probably reflects normal variations of meconium
consistency. Meconium plugs usually form in the lower end of the bowel because this meconium formed early
in intrauterine life and has the best chance to become dry and obstructive. The condition is associated with
Hirschsprung disease, cystic fibrosis, hypothyroidism, and magnesium sulfate administration to halt preterm
labor (Cuenca, Ali, Kays, et al., 2012).

Assessment

Because the obstruction is low in the intestinal tract, signs of obstruction such as abdominal distention and
vomiting may not occur for at least 24 hours. Typically, the infant will be identified first as an infant who has
had no meconium passage in the first 24 hours postbirth. A gentle rectal examination may reveal the presence of
hardened stool, although the plug may be too high up in the bowel to be palpated. An X-ray or sonogram may
reveal distended air-filled loops of bowel up to the point of obstruction. A barium enema not only reveals the
level of obstruction but also may be therapeutic in loosening the plug.

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Therapeutic Management

A subset of patients experiences spontaneous resolution of the meconium plug without any treatment. Of the
70% of patients requiring treatment, contrast or saline barium enema was used, with 97% success (Cuenca et
al., 2012). The administration of a small (about 5 ml) saline enema (never use tap water in newborns, infants,
and young children because it can lead to water intoxication) may cause enough peristalsis to expel the plug.
Instillation of acetylcysteine (Mucomyst) rectally may be prescribed to soften stool. Gastrografin, a highly
osmotic radiographic substance, administered as an enema, is yet another solution. Because it is hyperosmotic,
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the substance pulls fluid into the bowel, allowing the stool to soften and the plug to pass. Assess that the infant
is well hydrated before and after the procedure because an infant can become hypovolemic from the effect of
such a strong-acting medium.

Once the thickened portion of meconium has been passed, the infant should have no further difficulty and, over
the next several hours, may pass a great amount of stool. Postprocedure prognosis is very good. Observe the
infant for further passage of meconium (which should occur at least once daily) over the next 3 days, however,
to be certain additional plugs do not exist farther up in the bowel. If an infant is going to be discharged before
this time, instruct parents on the importance of observing for meconium and also about phoning their primary
care provider should the infant have no further bowel movements while at home.

Occasionally, a neonate passes a small plug of hardened meconium¡ªhard enough it would have caused an
obstruction except it is so small¡ªin the first 1 or 2 days of life. Be certain to record and report such a finding
because the infant will need close observation for continued defecation, the same as for the infant who actually
had an obstruction, to be certain there is not a larger and truly obstructing plug higher in the bowel.

Assess the family history of a newborn who has a meconium plug for cystic fibrosis, a recessively inherited
disorder (see Chapter 40), aganglionic megacolon (Hirschsprung disease), or a polygenic inherited disorder (see
Chapter 45) because, if there is a family history, the infant will need observation for these disorders.
Hypothyroidism can also present with constipation or hardened stool in newborns along with signs such as a
large protruding tongue, lethargy, and subnormal body temperature. Both hypothyroidism and cystic fibrosis
screening are done along with phenylketonuria screening in newborns. Be certain this blood test is obtained in
any newborn with a meconium plug (Levy, 2010). Infants born at home, especially, may not have had this done.

Meconium Ileus
Meconium ileus (obstruction of the intestinal lumen by hardened meconium) is a specific phenomenon that
occurs almost exclusively in infants with cystic fibrosis, resulting from the abnormal pancreatic enzyme
function seen with cystic fibrosis (see Chapter 40) and reflects extreme meconium plugging (Tappero &
Honeyfield, 2015). The usual symptoms of bowel obstruction occur: no meconium passage, abdominal
distention, and vomiting of bile-stained fluid. Meconium ileus is the only bowel obstruction that can present
with abdominal distention at birth (Tappero & Honeyfield, 2015). Unlike simple meconium plugging, the
obstruction point may be too high in the intestine for enemas to reduce it; instead, the bowel must be incised
and the hardened meconium removed by laparotomy. Meconium ileus is so strongly associated with cystic
fibrosis, the infant needs close follow-up by an interprofessional cystic fibrosis team in the following months.

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What If . . . On the second day of life you notice Maia Sparrow¡¯s baby, who was born with meconium
staining, is spitting up green mucus. Would it be safe to assume this is just more meconium-stained mucus?
Should the nurse consider the baby might be vomiting bile-stained vomitus?

Diaphragmatic Hernias
A diaphragmatic hernia is a weakness in the musculature that permits a portion of the abdominal organs, such as
the stomach or intestine, to protrude through the chest wall. This can cause a collapse of the left lung due to
cardiac displacement on the right side of the chest. It occurs in 1 to 5 per 10,000 live births, with slight
increased frequency in boys and a decreased frequency in Blacks (Oluyomi-Obi, Kuret, Puligandla, et al.,
2017).

The defect is caused because, early in intrauterine life, the chest and abdominal cavity are one; at approximately
week 8 of intrauterine growth, the diaphragm forms and divides them. If the diaphragm does not form
completely, the intestines can herniate through the diaphragm opening into the chest cavity as a diaphragmatic
hernia (Fig. 27.13). A diaphragmatic hernia. The bowel loop in the chest compresses the heart and lung on that
side.

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Assessment

Diaphragmatic hernia is frequently detected in utero by routine sonogram (Oluyomi-Obi et al., 2017). If not, it
is apparent at birth when the newborn has extreme difficulty establishing effective respirations. It can also be
indicated by a scaphoid (sunken) abdomen caused by the displacement of abdominal contents into the chest
(Tappero & Honeyfield, 2015). Surgeons have tried fetal surgery to correct or lessen the lung compromise from
this diagnosis, but multiple randomized trials comparing prenatal surgical intervention to postnatal intervention
show no benefit to prenatal intervention (Hedrick, 2013).

At birth, breath sounds are usually absent on the affected side of the chest cavity because at least one of the
lobes of the lungs on that side cannot expand completely (and may not have fully formed). The infant may be
cyanotic with intercostal or subcostal retractions. The abdomen generally appears sunken because it is not as
filled with intestine as usual. These infants have a potential for developing persistent pulmonary hypertension
because blood cannot perfuse readily through the unexpanded lung. This can lead to right-to-left shunting
through the foramen ovale in the heart and also causes the ductus arteriosus to remain patent. One condition,
then, has led to another until heart involvement complicates an already serious lung picture. The mechanics of
right-to-left heart shunts are further discussed in Chapter 41.

Therapeutic Management

Although surgical repair may be done as an imminent surgical procedure, it is usually delayed until
cardiorespiratory status has been stabilized as much as possible and the baby can be transported to a high-acuity
nursery for care. Surgery includes repair of the diaphragm and replacement of the herniated intestine and organs
back into the abdomen, possibly requiring both thoracic and abdominal incisions. If the disorder of the
diaphragm is large, an insoluble polymer (Teflon) patch may be used to reconstruct a better diaphragm shape.

The repair can be complicated if there is not enough room in the abdomen for the intestine to be returned. In
these infants, the abdominal incision may not be closed. The intestine is covered by silicone elastomer (Silastic)
and left to be closed at a later date after the abdomen has grown, the same as gastroschisis surgery.

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If the lung that was compressed in utero is truly hypoplastic and so cannot function, it will be removed at the
time of surgery. If it is developed but just deflated, over the next week after surgery, the lung will gradually
expand and begin to function. Chest tubes may not be used to avoid increased respiratory work and
overdistention of the compromised lung. The mortality rate of children with diaphragmatic hernia is about 40%,
with death often occurring because of associated anomalies of the heart, lung, and intestine and because of
premature birth (Kadir & Lilja, 2017).

Nursing Diagnoses and Related Interventions

Nursing Diagnosis: Risk for ineffective gas exchange related to displaced bowel

Outcome Evaluation: The baby appears stabilized with PO2 over 40 mmHg, PCO2 under 60 mmHg, and
pulmonary artery pressure at baseline level; lungs are clear to auscultation.

At birth, the infant is immediately endotracheally intubated and then gently ventilated with low-pressure, high-
frequency, low-intensity oxygen to aid breathing. A nasogastric tube or a gastrostomy tube will be inserted to
prevent the distention of the herniated intestine and to avoid further respiratory difficulty. Keep the infant NPO
to prevent the bowel from filling and becoming distended. Be certain the suction is attached to only low
intermittent suction to avoid injuring the lining of the newborn’s stomach.

While they wait for surgery, infants breathe better if they are turned on their side with the compressed lung
down and their head elevated because this allows the herniated intestine to fall back as far as possible into the
abdomen and allows the unaffected lung to expand more completely.

Following surgery, maintain the infant in a head elevated position to keep the pressure of the replaced intestine
off the repaired diaphragm. Suction the airway as necessary and keep the infant in a warmed, humidified
environment to encourage lung fluid drainage from the now uncompromised lungs. Chest physiotherapy may be
prescribed to ensure lung secretions do not pool and cause pneumonia. Positive- pressure ventilation may be
ordered to increase lung expansion, although this pressure is kept to a minimum to prevent tearing the
undeveloped or previously unopened lung tissue. Maintaining arterial oxygen (PO2) at a lower level of 60
mmHg and permitting PCO2 to rise to a higher level of up to 60 mmHg may help prevent damage to the
immature lung, thereby improving lung function (O Rourke-Potocki, Ali, Murthy, et al., 2016). In addition,
inhaled nitric oxide, or extracorporeal membrane oxygenation (ECMO) may be used as other ways to aid
respirations and increase oxygenation.

Nursing Diagnosis: Risk for imbalanced nutrition, less than body requirements, related to NPO status

Outcome Evaluation: Child’s skin turgor remains good; weight is maintained within 10% of birth weight or
between a percentile curve on growth chart.

After surgery, to prevent pressure on the suture line in the diaphragm by a full stomach and bowel, nutrition will
be supplied with TPN. When oral feedings are started, be certain to burp the infant frequently during and after
feedings to reduce the amount of swallowed air and limit bowel pressure against the diaphragm.

The nurse notices Mia remains obviously upset at her child’s appearance. She doesn’t want
to feed him and tells the nurse she’d like to place him for adoption.
In contrast, the child’s father, also a teenager, handles the baby warmly and asks
questions about surgery. Still no grandparents visit.
What interventions would the nurse want to begin with this family?
`

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